Emapalumab for the treatment of relapsed/refractory hemophagocytic lymphohistiocytosis

被引：125

作者：

Vallurupalli, Mounica ^{[1
]}

Berliner, Nancy ^{[1
]}

机构：

[1] Harvard Med Sch, Brigham & Womens Hosp, Dept Med, Div Hematol, 75 Francis St, Boston, MA 02115 USA

来源：

BLOOD | 2019年 / 134卷 / 21期

关键词：

MACROPHAGE ACTIVATION SYNDROME; CD8(+) T-CELLS; INTERFERON-GAMMA; MURINE MODELS; RUXOLITINIB; DISEASE; ANEMIA;

D O I：

10.1182/blood.2019002289

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Emapalumab is a fully human immunoglobulin G1 monoclonal antibody directed against interferon-gamma (IFN-gamma), which in November 2018 received the first global approval for the treatment of pediatric and adult patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance to HLH therapy. This review will highlight the pathophysiology of primary HLH, the therapeutic rationale for use of IFN-gamma-targeting therapy, and potential limitations to its broader use in the treatment of HLH.

引用

页码：1783 / 1786

页数：4

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